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Adolescent idiopathic scoliosis: Management and prognosis

Adolescent idiopathic scoliosis: Management and prognosis
Literature review current through: Jan 2024.
This topic last updated: Jul 14, 2023.

INTRODUCTION — Scoliosis, lateral curvature of the spine with associated rotation of the spinal column, is a structural alteration that occurs in a variety of conditions. Progression of the curvature during periods of rapid growth can result in significant deformity, which may be accompanied by cardiopulmonary compromise. Adolescent idiopathic scoliosis (AIS) is the most common type of scoliosis. Other types include congenital scoliosis, neuromuscular scoliosis, and syndromic scoliosis.

The treatment and prognosis of adolescent idiopathic scoliosis will be reviewed here. The clinical features, diagnosis, and initial evaluation are discussed separately. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis".)

TERMINOLOGY — AIS is scoliosis with Cobb angle >10° (image 1), age of onset ≥10 years, and no underlying etiology (eg, congenital, neuromuscular, syndromic). (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis".)

RISK FOR PROGRESSION — The risk for progression of AIS has implications for management. However, it is impossible to predict with complete accuracy which curves will progress and which will not [1].

Clinical predictors — Curves progress in approximately two-thirds of skeletally immature patients before they reach skeletal maturity [1,2]. Natural history studies indicate that the magnitude of progression is increased in:

Patients <12 years [3]

Females compared with males [3,4]

Premenarchal compared with postmenarchal females [5]

Curves with initial Cobb angle ≥20°, independent of age [6-9]

Double and thoracic curves compared with nonthoracic curves [1,3,9,10]

Risser grade 0 or 1 (figure 1) compared with Risser grade ≥2 [9]

Sanders skeletal maturity stage 3 or less compared with stage 4 or greater (figure 2) [11]

In a review of 123 skeletally immature patients (mean age 14 years) with idiopathic scoliosis and Cobb angles <50° who were followed without treatment until skeletal maturity, the average curve measured 33° (range 10 to 49°) at the time of diagnosis and 49° (range 12 to 97°) at skeletal maturity [2]. The curves progressed by:

<5° in 32 percent

≥5° in 68 percent

>10° in 34 percent

>20° in 18 percent

>30° in only 8 percent

Genetic testing — We do not use genetic testing (ie, the AIS prognostic test [AIS-PT], marketed as ScoliScore) to assess the risk for scoliosis progression. AIS is a complex disorder that appears to result from the interaction of multiple genetic loci and the environment, but the details of these interactions are not fully understood [12].

The AIS-PT is an algorithm developed to predict the risk of scoliosis progression to a Cobb angle >40° in skeletally immature White adolescents age 9 to 13 years who present with a Cobb angle of 10 to 25° [13]. The algorithm incorporates the initial Cobb angle and saliva-based deoxyribonucleic acid (DNA) testing for 53 genetic markers of severe scoliosis progression. The AIS-PT neither confirms nor excludes the diagnosis of AIS. Validation of ability to predict the risk of progression in independent cohorts of White adolescents and other ethnicity cohorts is lacking [14-17]. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Etiology'.)

Lack of validation of the AIS-PT in independent cohorts may be related to differences in the test population, genetic variability [17], or loss to follow-up of patients with nonprogressive scoliosis [14,18]. Until these issues are resolved, we continue to use clinical predictors rather than the AIS-PT to predict the risk for progression in patients with AIS.

INDICATIONS FOR REFERRAL — Indications for referral to an orthopedic surgeon for patients with AIS vary geographically. Our suggested indications for referral to an orthopedic surgeon are based on measurement of the angle of trunk rotation (ATR) using a scoliometer (picture 1) or determination of the radiographic Cobb angle (image 1) [19-21]. At the time of referral, it is important to specify whether the curve was measured by Cobb angle or scoliometer because the two measures are not directly equivalent. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Use of a scoliometer' and "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Cobb angle'.)

Our suggested indications for referral include:

ATR ≥7° in patients with body mass index (BMI) <85th percentile

ATR ≥5° in patients with BMI ≥85th percentile

Cobb angle between 20 and 24° in males or premenarchal females age 12 to 14 years

Cobb angle >25° in any patient

Progression of Cobb angle of ≥5° in any patient

Adolescents with idiopathic scoliosis and low risk for progression (eg, postmenarchal females; males age ≥15 years) may be followed by their primary care provider if the provider is comfortable doing so. However, referral to an orthopedic surgeon may be warranted if the patient or caregivers have questions that are beyond the scope of the primary care provider.

APPROACH TO SURVEILLANCE AND MANAGEMENT — Management is individualized according to the remaining growth potential, magnitude of the curve (Cobb angle or scoliometer measurement), best estimate of risk for progression, and patient and caregiver preferences. Individual patients and caregivers may place a different value on the potential risks and benefits of the various treatment options [22]. Our approach is described below. Other centers may use different thresholds for bracing or surgery.

Goal — The goal of the treatment is a curve with a Cobb angle of <40° at skeletal maturity. Natural history studies indicate that curves <40° have a lower risk for progression after skeletal maturity [6,23-26]. (See 'Outcome' below.)

Assessment of remaining growth — The Risser sign and Sanders skeletal maturity staging system are two methods of assessing remaining growth using radiographs.

For patients who have not had radiographs or in whom the Risser sign or Sanders stage cannot be determined, remaining growth potential can be estimated by a combination of chronologic age, height velocity, and sexual maturity rating (Tanner stage). Patients with substantial growth remaining generally are younger than 12 years, have not undergone their pubertal growth spurt, and have sexual maturity rating ≤2 (figure 3A-B).

Risser sign – The Risser sign (figure 1) has been traditionally used to estimate remaining growth potential in children with AIS. Most studies and recommendations in the past have been based on the Risser sign, which is assessed on posteroanterior (PA) spine radiographs:

Substantial growth remaining – Risser grade 0 to 2

Little growth remaining – Risser grade 3

Skeletal maturity – Risser grade 4 in females and Risser grade 5 in males

Sanders skeletal maturity staging system – Many practitioners prefer the Sanders staging system (figure 2) because it has greater correlation with peak height velocity and is better at predicting progression of scoliosis to surgery than the Risser sign [11,27].

Substantial growth remaining – Sanders stage 3 or less

Little growth remains – Sanders stage 4 to 7

Skeletal maturity – Sanders 7 to 8

Individualized management

Cobb angle 50 degrees or greater — Surgery should be discussed for patients with Cobb angles ≥50° at the time of presentation or later, regardless of skeletal maturity. (See 'Surgery' below.)

Curves with Cobb angles ≥50° at skeletal maturity may progress approximately one degree per year after cessation of growth [6,28]. Over 30 years, such curves may progress to Cobb angles >80°. Thoracic curves with Cobb angle ≥70 to 80° have been associated with compromised pulmonary function [24,29]. (See 'Outcome' below.)

Substantial growth remaining

Normal ATR rotation – Patients with substantial growth remaining and normal ATR as measured with a scoliometer (picture 1) can be followed clinically approximately every six months [30]. For patients with body mass index (BMI) <85th percentile, we consider <7° to be the normal ATR. For patients with BMI ≥85th percentile, we consider <5° to be the normal ATR [31]. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Use of a scoliometer'.)

Scoliosis radiographs (standing, full-length PA and lateral views of the spine [C7 to the sacrum and iliac crest]) should be obtained if the ATR increases to ≥7° in patients with BMI <85th percentile or if the ATR increases to ≥5° in patients with BMI ≥85th percentile. The Cobb angle determines subsequent management, as described in the sections that follow. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Radiographic evaluation'.)

Cobb angle 10 degrees or less – Curves with Cobb angle ≤10° do not meet the Scoliosis Research Society's definition of scoliosis (curves with Cobb angle >10°). The caregiver should be told that such a small curve is common and has no clinical significance; no follow-up other than routine screening at well-child visits is needed. Repeat imaging is not indicated unless there is clinical progression of the curve or significant pain (pain that limits activities or requires frequent analgesia).

Cobb angle 11 to 19 degrees – We recommend observation for patients with Cobb angles of <20° and Risser grade 0 to 2 or Sanders stage 1 to 3. Patients are followed clinically every six to nine months until skeletal maturity (six months when children are younger with more growth remaining; nine months when children are older with less growth remaining). (See 'Assessment of remaining growth' above.)

Curves <20° may progress approximately one degree per month during the adolescent growth spurt [32]. In a natural history study, among skeletally immature patients with a Cobb angle between 5 and 19°, the risk for progression (defined by increase of ≥10° with final curve ≥20°) was 22 percent if the Risser grade was ≤1 at the time of presentation and approximately 2 percent if the Risser grade was ≥2 [9].

Decisions about radiographs in such patients are made on a case-by-case basis. The limited added benefit of serial radiographs for observation of small curves must be balanced against the cost and cumulative radiation exposure. We obtain follow-up radiographs if there is an increase in the scoliometer reading or worsening clinical appearance of the curve.

If ordered, follow-up radiographs should include standing, full-length PA views of the spine [33]. The lateral view need not be repeated if the initial radiograph demonstrated the normal configuration of thoracic kyphosis and lumbar lordosis. (See "Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis", section on 'Radiographic evaluation'.)

If follow-up radiographs are obtained and the Cobb angle has progressed by ≥5° or measures ≥20°, bracing may be indicated, and a referral to an orthopedic surgeon should be made. (See 'Bracing' below.)

Cobb angle 20 to 24 degrees – We suggest initial observation for patients with Cobb angles of 20 to 24° and Risser grade 0 to 2 or Sanders stage 1 to 3. However, if there is documented progression or in the context of shared decision making, bracing is reasonable [34]. Some centers also suggest exercises to strengthen the back. (See 'Exercise therapy' below.)

Observed patients are followed clinically and often radiographically every four to six months until skeletal maturity (four months when children are younger with more growth remaining; six months when children are older with less growth remaining). (See 'Assessment of remaining growth' above.)

Bracing may be indicated if the Cobb angle increases by ≥5° over a three- to six-month period. In a natural history study, among skeletally immature patients with a Cobb angle 20 to 29°, the risk of progression of ≥5° was 68 percent if the Risser grade was ≤1 and 23 percent if the Risser grade was ≥2 [9].

Initial observation for skeletally immature patients with AIS and Cobb angle 20 to 29° is supported by the multicenter Bracing in Adolescent Idiopathic Scoliosis Trial (BrAIST), which compared thoraco-lumbar-sacral orthosis and observation in skeletally immature adolescents (10 to 15 years) with Cobb angle 20 to 40° [34]. Treatment success (defined by Cobb angle <50° at skeletal maturity) was achieved in 40 to 50 percent of patients assigned to observation or who wore their brace ≤6 hours per day [34].

Cobb angle 25 to 39 degrees – We suggest bracing for patients with Cobb angles of 25 to 39° and Risser grade 0 to 2 or Sanders stage 1 to 4. We monitor patients who are braced clinically and radiographically every five to six months until skeletal maturity (Risser grade 4 in females; Risser grade 5 in males; Sanders stage 7b). Surgical correction may be indicated if the curve progresses to ≥50° despite bracing or if the curve progresses rapidly (≥10° in one year).

Bracing does not correct curvature that is present at the time of diagnosis [35-37] but reduces the risk of curve progression to >50° at skeletal maturity (the usual threshold for surgery) [34]. The efficacy of bracing is directly related to the number of hours per day that the brace is worn. (See 'Bracing' below.)

In the multicenter BrAIST study, which compared thoraco-lumbar-sacral orthosis wear and observation in skeletally immature adolescents with Cobb angle 20 to 40°, more adolescents treated with bracing had Cobb angle <50° at skeletal maturity (72 versus 48 percent) [34]. Other types of braces, such as night time bending braces (eg, Charleston, Providence, etc) or strapping systems (eg, SpineCor) were not used in the BrAIST study and remain of unproven efficacy. (See 'Bracing' below.)

Cobb angle 40 to 49 degrees – We suggest either bracing or surgery for patients with substantial growth remaining (Risser grade 0 to 2) and Cobb angles between 40 and 50° at the time of presentation. We monitor patients who are braced clinically and radiographically every five to six months until skeletal maturity (Risser grade 4 to 5 for females; Risser grade 5 for males; Sanders stage 7b). Surgical correction may be indicated if the curve progresses to ≥50° despite bracing (See 'Bracing' below and 'Surgery' below.)

Little growth remaining — Patients at Risser grade 3 (figure 1) or Sanders stage 5 to 7 have little growth remaining and are not candidates for bracing. Management depends upon the magnitude of the curve at the time of presentation.

Cobb angle <40° – We suggest that patients at Risser grade 3 or Sanders stage 5 to 7 and Cobb angle <40° at the time of presentation be followed every 6 to 12 months until one year after skeletal maturity. For these patients, we determine skeletal maturity clinically: two years after menarche for females; shaving every day (or need to shave every day) for males.

Cobb angle 40 to 49° – We suggest observation for patients at Risser grade 3 or Sanders stage 5 to 7 and Cobb angle 40 to 49° at the time of presentation. Such patients are near the threshold for surgery but are not candidates for bracing. We follow them with radiographs every six to nine months until at least one year after skeletal maturity. For these patients, we determine skeletal maturity radiographically: Risser grade 4 in females, Risser grade 5 in males, or Sanders stage 8. These patients are at greater risk for progression than those with Cobb angle <40° at presentation and need to be followed more precisely. If the curve progresses to 50° before full skeletal maturity, surgery is indicated. In consultation with their orthopedic surgeon, some patients may opt for earlier surgery.

Skeletally mature patients — Skeletal maturity is defined by Sanders stage 8 or Risser grade 4 in females and Risser grade 5 in males.

Cobb angle <30 degrees – We provide reassurance for skeletally mature patients with AIS and Cobb angle <30° and discharge them from care. They do not need regular follow-up because their curves are not likely to progress [6,24]. (See 'Outcome' below.)

Cobb angle 30 to 49 degrees – We assess and manage skeletally mature patients with AIS and Cobb angle 30 to 49° on an individual basis according to patient preferences . Factors to be considered in the decision include the benefits and risks of spine surgery and the patient's concerns about appearance, risk of progression in adulthood (typically 1° per year [6,24]), and back pain in adulthood. (See 'Long-term follow-up' below and 'Outcome' below and "Scoliosis in the adult".)

TREATMENT MODALITIES — The main options for treatment of AIS include observation, bracing, and surgery [1,10,38,39]. Scoliosis-specific exercise therapy (eg, Schroth) is offered at some centers as an adjunct to observation or bracing. Although evidence for the efficacy of scoliosis-specific exercise therapy is limited [40,41], it is unlikely to be harmful. (See 'Exercise therapy' below.)

There is a lack of high-quality evidence from randomized trials that chiropractic treatment, electrical stimulation, or biofeedback is effective [42-44].

Observation — Observation is recommended for patients with AIS and Cobb angle <20° and is an option for patients with Cobb angle between 20 and 40°. Patients who are observed are followed clinically and/or radiographically until skeletal maturity or curve progression that requires bracing or surgery. The frequency of clinical and radiographic follow-up depends upon the severity of scoliosis and remaining growth potential. (See 'Substantial growth remaining' above.)

Bracing

Indications and contraindications

Indications – In skeletally immature patients with AIS, bracing reduces the risk of curve progression to ≥50° (the usual threshold for surgery) at skeletal maturity [34,45,46]. The efficacy of bracing is directly related to the number of hours per day that the brace is worn [47].

We suggest bracing for skeletally immature patients (Risser sign 0 to 2 or Sanders stage 1 to 3) whose Cobb angle is 25 to 39° at the time of presentation and skeletally immature patients whose Cobb angle is between 20 and 24° with documented progression ≥5° over any six- to nine-month period during observation. Our indications for bracing are consistent with those of the Scoliosis Research Society [48].

Bracing also is an option for skeletally immature patients with Cobb angle between 20 to 24° or 40 to 49° who choose bracing over observation or surgery, respectively.

In the multicenter Bracing in Adolescent Idiopathic Scoliosis Trial (BrAIST), which compared thoraco-lumbar-sacral orthosis and observation in skeletally immature adolescents (10 to 15 years) with Cobb angle 20 to 40°, more adolescents treated with bracing had Cobb angle <50° at skeletal maturity (72 versus 48 percent, odds ratio [OR], adjusted for nonrandom assignment 1.9, 95% CI 1.1-3.5) [34]. The proportion of patients who had Cobb angle <50° at skeletal maturity increased with increasing duration of brace wear (93 percent among those averaging ≥12.9 hours/day versus 41 percent among those averaging ≤6 hours/day).

The rate of brace failure appears to be increased in patients at Risser stage 0 (particularly those with open triradiate cartilage) and in patients with thoracic curves [49,50]. In an observational study of 168 patients who were treated with braces for AIS with Cobb angles of 25 to 45°, the rate of brace failure (ie, surgery or curve progression to ≥50°) was 44 percent in 120 patients at Risser stage 0, 7 percent in 29 patients at Risser stage 1, and 0 percent in 19 patients at Risser stage 2 [50]. The rate of brace failure was 34 percent in patients with thoracic curves and 15 percent in those with lumbar curves [49].

Contraindications – Contraindications to bracing include:

Little growth remaining or skeletal maturity (Risser grade 3 to 5 or Sanders stage 5 to 8)

Cobb angle ≥50°

Cobb angle <20°

Thoracic lordosis is a relative contraindication [51]. Some authors maintain that thoracic lordosis is a component of all structural scoliosis curves [52].

Types of braces — Most curves can be managed with an underarm brace (a thoraco-lumbar-sacral orthosis [TLSO], also known as the Boston brace) (picture 2). The TLSO is relatively easy to hide under clothing and fairly well accepted by most patients. Other types of underarm braces include the Charleston brace and the Providence brace, which are designed to be worn only at night [53-56]. These nighttime bending orthoses may be effective for isolated thoracolumbar or lumbar scoliosis curves with Cobb angles less than 35° [57]. A nonrigid brace (SpineCor), which consists of a series of straps, is also available [58]. Data regarding the efficacy of the different types of braces are mixed; differing inclusion criteria preclude direct comparisons [59-61].

A small percentage of curves require a brace with an under-chin extension (a cervico-thoraco-lumbar-sacral orthosis [CTLSO], also known as the Milwaukee brace). The CTLSO is more difficult to hide under clothes and less well-tolerated by patients. Indications for CTLSO bracing include thoracic curve with apex at or above T8, and double thoracic curves (high-left and lower-right thoracic curve pattern and right thoracic/left thoracolumbar pattern) [10].

Patient instructions — We instruct the patient to wear the brace 18 hours per day [50], acknowledging that 13 hours may be adequate [34]. Many sports can be played while wearing the brace, but it may be removed for sports that cannot be performed while wearing it (eg, swimming, gymnastics).

In the past, wearing the brace 23 hours per day was recommended. However, in the BrAIST trial, 93 percent of patients who wore their brace an average of ≥12.9 hours per day had a successful outcome (ie, Cobb angle <50° at skeletal maturity) [34]. Patients at Risser stage 0 may benefit from increased time wearing their brace [50].

Adherence to brace wear can be objectively and quantifiably monitored with temperature or pressure sensors embedded in the brace. Most commercially available braces can be implanted with such sensors, and we offer the caregiver of the option of having one implanted. Several studies demonstrate that patients may not adhere to the bracing schedule and patient-reported brace wear may be overestimated [34,62-64]. In observational studies that monitored adherence (eg, with a temperature sensor), braces were worn for an average of 65 percent of the prescribed hours [62,63]. Awareness of monitoring and counseling may increase adherence [65]. In a small randomized trial, adherence to an 18-hour schedule was greater among patients who were told that their adherence was being monitored with a temperature sensor than among those who were not told (85.7 percent versus 56.5 percent) [66].

Monitoring and discontinuation

Monitoring – Patients who are treated with braces should be seen shortly after the first fitting for a radiograph in the brace to make sure that the brace fits appropriately [38]. A properly fitting brace will improve the Cobb angle while the brace is being worn. The goal in-brace correction is at least 20 percent improvement in Cobb angle compared with the prebrace measurement (eg, from 35 to <28°). Cobb angle correction of at least 20 percent has been associated with prevention of curve progression [67]. The family needs to understand that the in-brace correction is only transitory while the brace is being worn and the Cobb angle will return to the original curve magnitude (assuming no progression) with subsequent out-of-brace radiographs. The author of this topic review typically obtains the first in-brace radiograph to determine curve-correction after four weeks, but there is no accepted standard. Some surgeons prefer to get the initial in-brace radiograph within a few days of the first fitting.

After the initial in-brace radiograph, we monitor brace treatment clinically and radiographically every five to six months. During these visits, brace fit should be assessed and radiographs (in or out of brace) should be obtained to assess curve progression [38]. There is no consensus on the best way to monitor the scoliosis in patients wearing a brace. If obtaining out-of-brace radiographs, we generally ask the patient to remove the brace the night before the study, but there is no accepted standard. In a small observational study, maximum change in Cobb angle was achieved after at least 120 minutes out of the brace [68].

Progression of the Cobb angle of ≥5° despite adequate bracing is a poor prognostic sign that surgery may be necessary. If documented curve progression is observed, the brace should be carefully assessed to be sure it still fits properly, and adherence to the bracing schedule should be confirmed. (See 'Surgery' below.)

Discontinuation – Brace use should be continued until the end of growth, typically one to two years postmenarche and Sanders stage 7b or Risser grade 4 to 5 for females and Risser grade 5 for males. Some practitioners wean brace usage to nighttime (sleeping) only, and some simply discontinue it. However, patients often discontinue use on their own. Some centers suggest exercise therapy to maintain trunk strength while weaning from the brace. (See 'Exercise therapy' below.)

Patients typically have clinical and radiologic follow-up six months after discontinuation of the brace and then yearly for several years. There is often a several-degree increase of the curve in the months just following discontinuation of the brace, which quickly stabilizes. However, if the Cobb angle progresses to ≥50° surgery may be warranted.

Adverse effects — Adverse effects of bracing may include psychosocial effects (eg, diminished self-esteem, disturbed peer relationships), skin irritation, disturbed sleep, restriction of physical and recreational activities, and difficulty finding clothes that fit properly [40,69-71]. Studies demonstrating the frequency of these effects are lacking [70]. There is a single case report of compressive injury to the dorsal scapular nerve resulting in scapular winging in 15-year-old female after incorrect use of a thoracolumbar orthosis [72]. In a longitudinal radiographic study of 265 patients, underarm bracing was associated with flatback deformity (ie, reduced thoracic kyphosis and lumbar lordosis) but was not associated with reduced quality of life [73].

Surgery — The primary goal of surgical treatment of AIS is prevention of curve progression through spinal fusion, but there is limited evidence regarding this outcome. Successful fusion occurs in approximately >95 percent of cases [74,75].

Secondary goals include curve correction and improved quality of life. In a meta-analysis, the average coronal curve correction ranged from 48 to 67 percent with posterior instrumentation and 71 to 93 percent with anterior instrumentation [76]. In a multicenter study of the outcomes of surgical treatment, patients reported improved self-image, function, and level of activity on validated measures two years after surgery for AIS [77].

Indications and procedures

Indications – Surgical correction is indicated for skeletally immature patients with curves with Cobb angle ≥50° and some skeletally immature patients with Cobb angle between 40 and 50°. Surgical correction also is an option for skeletally mature patients with Cobb angle ≥50°. Surgery also may be warranted for patients with lumbar curves with marked trunk shift. (See 'Cobb angle 50 degrees or greater' above and 'Substantial growth remaining' above.)

Procedures – Procedures for correction of scoliosis involve spinal fusion, which may be performed posteriorly or anteriorly. Achieving bony fusion is the most important aspect of surgery, and either autograft or allograft may be used [78]. The off-label use of bone morphogenic protein (a group of purified proteins that stimulate bone growth) rather than autograft or allograft is not routinely indicated. The selection of fusion levels should be individualized to the patient and is based on the curve pattern, curve size, flexibility, and sagittal alignment (presence or absence of kyphosis) [79].

Posterior spinal fusion with instrumentation and bone grafting – Posterior spinal fusion (PSF) with instrumentation and bone grafting is the most common surgical procedure for AIS.

Contemporary implants for PSF are "segmental": a variety of hooks, screws, and wires are used to attach contoured rods to the spine at multiple vertebrae, or "segments" (image 2). The original implants for PSF were straight stainless steel rods connected to the cephalad and caudad regions of the spine with simple hooks (Harrington rods). Segmental instrumentation gives the surgeon greater control over the position and rotation of the spine [80]. The increased stability of segmental instrumentation permits early mobilization with ambulation the day after surgery without external support such as a body cast or brace. Newer generation implants that use titanium with or without cobalt chrome are more compatible with magnetic resonance imaging (MRI) than stainless steel implants.

Anterior spinal fusion with instrumentation and bone grafting – Anterior spinal fusion (ASF) with instrumentation and bone grafting may be performed for thoracolumbar and lumbar scoliosis. The convex side of the spine is exposed anteriorly (often with the assistance of a general surgeon) by a thoracotomy and/or retroperitoneal approach. The curve is corrected by shortening the convex side of the deformity.

The purported advantages of the anterior approach include less blood loss, lower risk of neurologic injury (because of correction by shortening instead of distraction), and no disturbance of the paraspinal muscles. Disadvantages include increased complexity and decreased pulmonary function if the thoracic cavity is entered and/or the diaphragm is opened [81].

With improvements in PSF instrumentation and techniques, there are relatively few indications for isolated ASF and it is rarely performed [82]. In a retrospective comparison, patients treated with posterior instrumentation and fusion for lumbar curves had better outcomes and shorter hospital stays than those treated with anterior instrumentation and fusion [83].

Thoracoscopic ASF – Anterior instrumentation of the thoracic spine may be performed thoracoscopically. The potential advantages of video-assisted thoracoscopic ASF over PSF with thoracic pedicle screws include reduced blood loss, fewer total levels fused, and the preservation of nearly one caudad fusion level. The disadvantages include increased operative time and slightly less improvement in pulmonary function [84]. Thoracoscopic anterior instrumentation is technically demanding and applicable to a limited number of curves. Its use seems to be decreasing [85].

Combined ASF and PSF – In patients with open triradiate cartilages, it is rarely necessary to perform an ASF, as well as a PSF, to prevent the "crankshaft phenomenon," in which the anterior spine continues to grow after the PSF, causing a severe rotational and sagittal alignment deformity [86,87]. However, there is some evidence to suggest that modern segmental instrumentation systems make anterior fusion unnecessary [88].

Growth modulation techniques – Growth modulation techniques include vertebral body tethering and unilateral periapical distraction. These techniques are minimally invasive, preserve motion, and do not preclude spinal fusion if they are unsuccessful.

-Vertebral body tethering – Vertebral body tethering devices are intended to gradually correct scoliosis by slowing growth on the convex side of the curve (image 3) [89]. A single tethering device has been approved by the US Food and Drug Administration (FDA) for the correction of idiopathic scoliosis that has not responded to conservative treatment options (eg, bracing) [90].

In observational studies, vertebral body tethering has been associated with progressive correction and appear to be safe, though overcorrection is a concern, and few long-term results are available [90-98]. Additional adverse effects include tether breakage and pneumothorax [90]. In one study, vertebral body tethering with staples was not effective for thoracic curves with Cobb angle ≥35° [92]. In a retrospective review of 17 skeletally immature patients who underwent vertebral body tethering (VBT) for scoliosis (14 for AIS) and were followed for two to four years, seven required revision surgery (removal of the tether for complete correction or overcorrection in four; addition of lumbar tether in one; replacement of a broken tether in one; and revision to PSF in one) [94]. In a retrospective study that compared outcomes two to five after VBT or PSF in patients with mean preoperative Cobb angle of approximately 53°, PSF was associated with greater correction (residual curve with Cobb angle of 16 versus 33°) and lower rates of revision surgery (0 of 26 for PSF and 9 of 23 for VBT) [99].

-Unilateral periapical distraction implant – A unilateral periapical distraction implant (minimally invasive deformity correction system, posterior dynamic deformity correction device) has been approved by the FDA as a humanitarian use device for the treatment of AIS [100]. This device is implanted on the concave side of the curve (image 4); it stabilizes and corrects the deformity through incremental ratchet lengthening that is activated by exercise [101,102]. Although initial reports of its use were promising [101,103], in a prospective cohort study, one center noted serious complications (eg, osteolysis, implant failure) in 10 of 20 patients and terminated the study early [104].

Additional studies are necessary to determine which patients are likely to benefit from growth modulation techniques, to better understand and refine the modulation technique (eg, force necessary to slow growth, tensile strength), and to see whether and how the concave side of the curve progresses over time [95].

Preoperative evaluation — The preoperative evaluation for scoliosis surgery includes standing posteroanterior (PA) and lateral spinal radiographs and pulmonary function tests for patients with curves ≥70 to 80°. Lateral bending radiographs (either supine or standing) are obtained for surgical planning to assess whether the curves are structural based on their flexibility. The Lenke classification is a surgical planning classification system used to help the surgeon select levels for surgery [79]. It is based on standing PA, standing lateral, and left/right bending radiographs. The classification system describes six curve patterns with subtypes based on lumbar curve and sagittal plane modifiers: main thoracic, double thoracic, double major (thoracic curve is larger), triple major, thoracolumbar, and double major (lumbar curve is larger) curve type.

Many hospitals require a preoperative pregnancy testing in postmenarche females or females beyond a certain age. Hypersensitivity reactions to metallic spinal implants are rare, and there is no consensus on the utility of testing preoperatively [105]. Scoliosis surgery is associated with an increased risk of blood loss. Pretransfusion blood testing and preoperative autologous blood donation are discussed separately. (See "Pretransfusion testing for red blood cell transfusion" and "Surgical blood conservation: Preoperative autologous blood donation".)

Complications — Complications of surgery include blood loss, infection, implant failure, neurologic injury including paralysis and blindness, progression of deformity around the fusion, and pseudoarthrosis (failure of fusion). Intraoperative blood salvage ("cell saver") and antifibrinolytics (epsilon aminocaproic acid and tranexamic acid) may be used to reduce blood transfusion requirements [106-109]. (See "Surgical blood conservation: Intraoperative blood salvage" and "Intraoperative use of antifibrinolytic agents" and "Overview of topical hemostatic agents and tissue adhesives", section on 'Hemostatic agents'.)

In a review of data from the Scoliosis Research Society Morbidity and Mortality database, the overall complication rate among patients who underwent surgery for AIS between 2004 and 2016 was 1.5 percent [110]. The complication rate decreased from approximately 5 to 1 percent between 2004-to 2007 and 2013 to 2016. The most common complications were surgical site infection, new neurologic deficit, and implant-related complications (each <0.6 percent). In an earlier multicenter study, 73 percent of patients with neurologic complications recovered completely and 22 percent recovered partially [111].

Long-term surgical complications were described in a retrospective review of patients treated for AIS who were followed for 22 to 23 years [75]. Among those who underwent surgery, the average curve deterioration was 3.5°; pseudoarthrosis developed in 1.9 percent and loss of lumbar lordosis in 2.6 percent; 5.1 percent needed additional surgery related to implants. The risk of these complications is expected to be lower with contemporary implants.

Postoperative care

Monitoring neurologic function – Neurologic function must be monitored closely for 48 hours after surgery because delayed neurologic injury may occur [112]. However, modifications in devices, techniques, and intraoperative monitoring have reduced the risk of catastrophic complications [82,113,114].

Return to activity – Recommendations for resuming sports participation vary by surgeon, from as little as 4 months to 12 months postoperatively [38,115,116]. We suggest approximately six months. Once the spine has fused, all sports are permitted, with the possible exception of collision sports (eg, football, hockey, rugby). The reduced motion of the fused spine may make it more difficult to perform activities such as gymnastics and dancing at a high level.

Considerations related to implanted metal – Antibiotic prophylaxis prior to dental procedures is not necessary following instrumentation for scoliosis [117].

MRI studies can be safely performed following spinal instrumentation for scoliosis, although some artifact is produced. The amount of artifact depends on the composition of the implants; greater artifact has been noted with stainless steel than with titanium alloys. Routine removal of spinal implants used in correction of scoliosis is not usually performed.

Exercise therapy — Some centers suggest physical or exercise therapy as an adjunct to observation or bracing, although the indications, objectives, and regimens vary.

Scoliosis-specific exercises (eg, Schroth, Scientific Exercises Approach to Scoliosis) generally are designed to provide three-dimensional self-correction, train activities of daily living, and stabilize corrected postures [41,118]. Others exercise programs may be designed to strengthen the back/core.

Although some randomized trials suggest that physiotherapeutic scoliosis-specific exercises may reduce the Cobb angle and improve health-related quality of life [119-121], systematic reviews of randomized and controlled clinical trials have concluded that the benefits are unproven [40,41]. Exercise therapy is unlikely to be harmful, but it may be time consuming.

LONG-TERM FOLLOW-UP — Follow-up with the orthopedic surgeon typically is scheduled for one to two years after the onset of skeletal maturity (Risser 4 in females, Risser 5 in males), regardless of treatment. The frequency and duration of subsequent orthopedic follow-up depends upon the magnitude of the curve at skeletal maturity and patient preference. All patients should receive regular primary care during adulthood for clinical monitoring of asymmetry/curve progression and quality of life.

We generally do not suggest routine orthopedic follow-up during adulthood for most skeletally mature individuals with curves with Cobb angle ≤30° and/or successful spinal fusion. Curves in such patients are unlikely to progress during adulthood [6,23,24].

We assess and manage skeletally mature scoliosis patients with Cobb angles between 30 and 49° on an individual basis (regardless of treatment) according to patient preference. Some patients prefer to be seen by an orthopedic surgeon periodically (eg, every one to five years); others prefer to be seen only as needed. Factors to be considered in the decision include the potential effects on quality of life (eg, concerns about appearance, back pain in adulthood) [122], the risk of progression in adulthood, and the risks and benefits of spine surgery (which may vary with age and type of procedure). (See 'Outcome' below and "Scoliosis in the adult".)

Skeletally mature patients with curves with Cobb angle ≥50° who were treated without surgery should be offered regular follow-up with their orthopedic surgeon. Curves of this magnitude are likely to progress in adulthood. (See 'Cobb angle 50 degrees or greater' above.)

We do not obtain serial radiographs for asymptomatic patients following surgical correction of AIS. In a series of 451 consecutive patients who had surgical correction of scoliosis (73 percent with AIS), findings on routine radiographs rarely altered clinical management in patients who were asymptomatic [123]. However, some surgeons obtain infrequent follow-up radiographs, typically at the first postoperative visit, then at one- and two-year follow-up visits, and then as needed if the patient presents with signs or symptoms indicative of a postoperative problem.

OUTCOME — Several studies have examined the long-term outcomes of AIS treated with observation, bracing, or surgery. Because surgical methods have changed over time, the longer-term outcomes for patients treated surgically reflect procedures/implants that are no longer used. Twenty-year follow-up is available for patients treated with Harrington rods, but few studies with more than 5- to 10-year follow-up are available for patients with segmental instrumentation [124-127].

Curve progression – Scoliosis can continue to progress after skeletal maturity in untreated patients. The risk of progression increases with increasing magnitude of the curve. Curves >50° generally progress one degree per year after skeletal maturity. Curves measuring ≤30° at the end of growth typically do not progress [6,23-26]. In a long-term study, 133 untreated curves in 102 patients were followed for an average of 40.5 years after skeletal maturity [6]. Two-thirds of the curves progressed, with an average rate of 0.75 to 1° per year. Factors related to progression after skeletal maturity included severity of curve at diagnosis (curves with Cobb angles >30° tended to progress, whereas smaller curves did not) and curve location (increased risk with thoracic curves).

Curves also may progress after bracing or surgery. In one study, patients who were treated with surgery or bracing were followed for an average of 22 to 23 years [75]. Average Cobb angle progression for patients treated with bracing and followed for 22 years was 7.9°. Average Cobb angle progression for patients treated surgically and followed for 23 years was 3.5°. In another observational study in 203 patients with thoracic scoliosis treated with segmental instrumentation, surgical outcomes were maintained for at least five years postoperatively (mean Cobb angle 16°, range -2 to 34°) [128].

Back pain – Follow-up studies indicate an association between AIS (treated or untreated) and mild or moderate back pain or degenerative disc changes in adulthood [24,26,75,129-137]. Some of the studies suggest a correlation between the initial magnitude of the curve and subsequent complaints of back pain [133,134]. Despite an increased risk of back pain, most patients with AIS have little, if any, functional limitation in adulthood, and the risk of neuropathy is low [26,53,129,135].

Psychosocial – Patients with AIS may have increased concerns related to body development and peer interactions, and decreased perception of health status, even after treatment [133,136,138,139]. In a review of outcomes 10 years after treatment for AIS, patients had moderately reduced perceived health status and activities of daily living compared with age-matched controls [133]. The patients treated surgically were less affected than those treated with bracing. In another study, at an average of 22 years after nonsurgical treatment for AIS, the perception of handicap was similar between AIS patients and controls [135].

Pregnancy – A systematic review of 22 observational studies (>3125 patients) concluded that most females with AIS are able to have children and do not appear to be at increased risk for pregnancy-related complications, although they have slightly increased rates of nulliparity, infertility treatment, and pregnancy related back pain [140].

Mortality – In long-term follow-up studies of patients with AIS, the mortality rate is not increased compared with that of the general population [24,129]. Increased mortality from cor pulmonale and right heart failure is seen only in patients with severe thoracic curves (>90 to 100°) [24,129,141].

Breast cancer – The risk of breast cancer in women exposed to diagnostic levels of radiation is discussed separately. (See "Factors that modify breast cancer risk in women", section on 'Exposure to diagnostic radiation'.)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Idiopathic scoliosis in adolescents".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient education" and the keyword[s] of interest.)

Basics topic (see "Patient education: Scoliosis (The Basics)")

SUMMARY AND RECOMMENDATIONS

Terminology – Scoliosis is defined as curvature of the spine in the coronal plane (image 5) with associated rotation of the spinal column. Adolescent idiopathic scoliosis (AIS) is scoliosis with Cobb angle >10° (image 1), age of onset ≥10 years, and no underlying etiology (eg, congenital, neuromuscular, syndromic). (See 'Terminology' above.)

Risk for progression – Curves progress in approximately two-thirds of skeletally immature patients before the patient reaches skeletal maturity. The magnitude of progression is increased in patients younger than 12 years, females compared with males, females who are premenarchal, curves with initial Cobb angle ≥20°, thoracic curves, double curves, patients at Risser grade 0 or 1 (figure 1), and Sanders stage 1 to 3 (figure 2). (See 'Risk for progression' above.)

Indications for referral – Our suggested indications for referral to an orthopedic surgeon for AIS include:

Angle of trunk rotation (ATR) as measured with a scoliometer ≥7° in patients with body mass index (BMI) <85th percentile or ATR ≥5° in patients with BMI ≥85th percentile

Cobb angle between 20 and 24° in premenarchal females or males age 12 to 14 years

Cobb angle >25° in any patient

progression of Cobb angle ≥5° in any patient

Adolescents with AIS and low risk for progression (ie, postmenarchal females, males older ≥15 years) may be followed by their primary care provider if the provider is comfortable doing so. (See 'Indications for referral' above.)

Treatment goal and approach to management – The goal of the treatment of AIS is a curve with a Cobb angle of <40° at skeletal maturity. Options for treatment include observation, bracing, and surgery. Management is individualized according to the magnitude of the curve (Cobb angle or scoliometer measurement), remaining growth potential, best estimate of risk for progression, and patient and caregiver preferences. (See 'Approach to surveillance and management' above.)

Surgery should be discussed for patients with Cobb angles ≥50° at the time of presentation or later, regardless of skeletal maturity. (See 'Cobb angle 50 degrees or greater' above and 'Surgery' above.)

For patients with substantial growth remaining (eg, Risser grade 0 to 2 or Sanders stage 1 to 3 at the time of presentation (figure 1)), our approach is as follows (see 'Substantial growth remaining' above):

-For patients with Cobb angles of 11 to 19°, we recommend observation (Grade 1A). Patients are followed every six to nine months until skeletal maturity. Bracing may be indicated if the Cobb angle increases by ≥5° or progresses to ≥20° during observation. (See 'Observation' above.)

-For patients with Cobb angles of 20 to 24°, we suggest observation (Grade 2B). Patients are followed clinically every four to six months until skeletal maturity. Bracing may be indicated if the Cobb angle increases by ≥5° over a three- to six-month period. (See 'Observation' above.)

-For patients with Cobb angles of 25 to 39°, we suggest bracing (Grade 2B). We monitor patients clinically and radiographically every five to six months until skeletal maturity. Surgery may be indicated if Cobb angles progress to ≥50° during bracing. (See 'Bracing' above.)

-For patients with Cobb angles between 40 and 49° and Risser grade 0 to 2 or Sanders stage 1 to 3 at the time of presentation, we suggest bracing, although surgery may be considered above 45° (Grade 2C). (See 'Bracing' above and 'Surgery' above.)

Patients at Risser grade 3 or above or Sanders stage 4 to 8 (figure 1) have little growth remaining and are not candidates for bracing. Management depends upon the magnitude of the curve at the time of presentation. (See 'Little growth remaining' above and 'Skeletally mature patients' above.)

Follow-up – Follow-up with the orthopedic surgeon typically is scheduled for one to two years after the onset of skeletal maturity (Risser 4 in females, Risser 5 in males), regardless of treatment. The frequency and duration of subsequent orthopedic follow-up depends upon the magnitude of the curve at skeletal maturity and patient preference. All patients should receive regular primary care during adulthood for clinical monitoring of asymmetry/curve progression and quality of life. (See 'Long-term follow-up' above and "Scoliosis in the adult".)

Outcome – Outcome for patients with AIS is generally favorable, whether they are treated with observation, bracing, or surgery. They have a slightly increased risk of back pain and degenerative disc changes compared with patients without AIS but have no increased risk of mortality or adverse pregnancy outcome. (See 'Outcome' above.)

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Topic 6291 Version 46.0

References

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